Generalised smooth-muscle disease with defective muscarinic-receptor function.
A patient with widespread smooth-muscle disease presented with chronic intestinal pseudo-obstruction but had in addition defects of the bladder, pupils, sweating, and cardiovascular function. There was no evidence of a primary neural lesion, and minor changes in the muscle did not resemble those of a myopathy. In each organ affected muscarinic cholinergic function was at fault, but instead of supersensitivity to cholinergic drugs, which occurs in postganglionic autonomic neuropathies, there was a lack of response to cholinergic drugs and anticholinesterases. It was therefore concluded that the patient had a new type of defect of muscarinic-receptor function. The cause was unknown, but it may have been an autoimmune disease resembling myasthenia, in which there is a postjunctional defect of muscarinic receptors. In similar cases binding of muscarinic agonists and antagonists should be tested. When antibodies to purified human muscarinic receptors become available different patterns of smooth-muscle defect may be identifiable, enabling the lesion to be defined more precisely.
D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000209:etiology; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000209:etiology / Q000473:pathology; D007583:Jejunum / Q000473:pathology; D009130:Muscle, Smooth / Q000473:pathology*; D009135:Muscular Diseases / Q000150:complications / Q000473:pathology*; D011950:Receptors, Cholinergic / Q000502:physiology*; D011976:Receptors, Muscarinic / Q000502:physiology*
D011950:Receptors, Cholinergic; D011976:Receptors, Muscarinic